Mad cow disease added to alert list

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Fears about the inadvertent spread of the human form of mad cow disease through the blood supply, transplantation or contaminated surgical instruments has prompted Australian health departments to make Creutzfeldt-Jakob disease (CJD) and its beef-related variant, vCJD, notifiable diseases for the first time.

Smallpox, despite its absence since 1979, and a rare but potentially fatal bacterial infection called tularaemia are now also notifiable because of their potential use in a bioterrorism attack.

Over the past month all NSW medical practitioners, hospital general managers and laboratories have been advised to notify their local public health unit of any instance of the diseases, although there have been none so far.

Other states have begun the same procedure as part of a national surveillance process.

The director of the NSW Health Communicable Diseases Branch, Dr Jeremy McAnulty, said the notifications, which were gazetted several months ago, were "a safety-net process" that enabled immediate investigation should a case arise.

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There are now 14 notifiable infectious conditions in NSW including SARS, AIDS, leprosy, syphilis, tuberculosis and acute viral hepatitis.

While it is hoped smallpox will not re-emerge, with limited samples stored in facilities in Russia and the US, there are fears of unauthorised stockpiles by extremist groups.

The potential for vCJD to emerge in Australia via a migrant or former resident of Britain particularly, or Europe, is greater.

Professor Colin Master, head of the Australian National CJD Case Registry, based at the University of Melbourne, said making CJD and vCJD notifiable would help the registry - which for the past decade had relied on referrals from pathologists and neurologists - to "monitor cases of CJD and their occurrence in Australia, follow demographic trends and identify infection-control issues that need to be managed".

CJD is believed to be infectious and transmittable even in the sometimes decades-long latency period before symptoms appear.

Eight months ago, the world's first possible transmission of vCJD via a blood transfusion was reported. After a second possible case was reported in The Lancet earlier this month, both the Federal Government's Special Expert Committee on Transmissible Spongiform Encephalopathies and the Australian Red Cross Blood Service reassessed contingency plans for vCJD.

While extremely rare, all types of CJD are fatal. Its newest strain, vCJD, has already infected 147 Britons as well as former British residents in Canada, the US and Hong Kong.The disease was first reported in 1996, after a decade of British government denials that the epidemic of bovine spongiform encephalopathy (BSE) in cattle would emerge in humans who had eaten contaminated beef products.

Most of the seven French vCJD victims to date are believed to have been infected locally as a result of the spread of BSE to France from the early 1990s. An Italian woman from Sicily who died last year of vCJD was thought to have spent time in a BSE-affected region of France.

BSE, which spread to central Europe through the export of contaminated meat-and-bonemeal protein supplements for cows, has moved into eastern Europe and Japan. The US and Canada each reported a case last year, raising fears of an outbreak hidden by low BSE testing rates.